Early focality and spread of cortical dysfunction in amyotrophic lateral sclerosis: A regional study across the motor cortices

•Cortical dysfunction was explored from multiple motor regions across hemispheres in ALS patients.•Early cortical dysfunction was focal and asymmetric, mirroring asymmetrical clinical onset.•Global dysfunction was apparent in later disease, with regional differences linked to clinical heterogeneity....

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Published in:Clinical neurophysiology 2020-04, Vol.131 (4), p.958-966
Main Authors: Dharmadasa, Thanuja, Matamala, José M., Howells, James, Vucic, Steve, Kiernan, Matthew C.
Format: Article
Language:English
Subjects:
Amyotrophic lateral sclerosis
Amyotrophic Lateral Sclerosis - physiopathology
Asymmetry
Clinical heterogeneity
Cortical dysfunction
Disease onset
Evoked Potentials, Motor - physiology
Female
Functional Laterality - physiology
Humans
Male
Middle Aged
Motor Cortex - physiopathology
Neural Conduction - physiology
Transcranial Magnetic Stimulation
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Summary:•Cortical dysfunction was explored from multiple motor regions across hemispheres in ALS patients.•Early cortical dysfunction was focal and asymmetric, mirroring asymmetrical clinical onset.•Global dysfunction was apparent in later disease, with regional differences linked to clinical heterogeneity. To characterise the regional cortical patterns underlying clinical symptomatology in amyotrophic lateral sclerosis (ALS). 138 patients prospectively underwent transcranial magnetic stimulation studies from hand and leg cortical regions of each hemisphere, obtaining motor evoked potentials from all four limbs. Patients were categorised by clinical phenotype and underwent clinical and peripheral evaluation of disease. Cortical dysfunction was evident across the motor cortices, with reduction in short-interval intracortical inhibition (SICI) suggesting the presence of widespread cortical hyperexcitability, most prominently from clinically affected regions (hand p 
ISSN:1388-2457
1872-8952
DOI:10.1016/j.clinph.2019.11.057